POS1463 FACTORS ASSOCIATED WITH DISEASE DAMAGE AT 20 YEAR FOLLOW-UP IN AN ITALIAN MULTICENTER SJÖGREN’S SYNDROME COHORT
Authors: G. Cafaro, F. Ferro, R. Dal Pozzolo, G. Fulvio, L. Bruno, S. Fonzetti, G. La Rocca, B. Dei, S. Falaschi, C. Perricone, R. Gerli, C. Baldini, E. Bartoloni
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Sjögren's syndrome (SS) represents a chronic autoimmune disorder characterized by a relatively benign course. However, up to 40% of SS patients develop systemic extra-glandular involvement which, in turn, may lead to long-term damage accrual. Few studies assessed damage accrual in SS with a follow-up ranging from 5 to 10 years. Knowledge of long-term risk of damage and awareness of factors contributing to damage accrual in SS represent important issues to identify patients requiring a closer follow-up and to better stratify therapy. <h3>Objectives</h3> To examine disease damage progression every 5 years until 20 years of follow-up and disease related features associated with higher damage score at 20 years in a multicenter SS cohort. <h3>Methods</h3> A cohort of 252 SS patients fulfilling the 2002 AECG and/or 2016 ACR-EULAR classification criteria was retrospectively analysed. The following variables were collected at diagnosis and every 5-year timepoints until 20 years: demographic, clinical and serologic parameters; disease activity by ESSDAI; Charlson Comorbidity Index (CCI); disease damage by SSDDI and treatments. Patients were grouped according to damage degree at 20 years: "low/no damage", LND (SSDDI ≤ 1) and "moderate/high damage", MHD (SSDDI ≥ 2). Continuous variables were compared by Mann-Whitney U test and categorical variables by Chi-squared test of Fisher's exact test, as appropriate. Significant variables at univariate analysis were include in a multivariable logistic regression analysis model with wild bootstrapping to account for collinearity and heteroscedasticity. <h3>Results</h3> A total of 50 patients (mean age at diagnosis 44±12 years, mean disease duration 24±5 years) had 20 year follow-up. Mean SSDDI increased from 1.2 (±1.5) at 5 to 1.5 (±1.7) at 10 years and from 1.54 (±1.7) at 15 to 2.04 (±2.2) at 20 years. Oral/ocular damage, peripheral nervous (PN) involvement and lymphoproliferative disease mainly contributed to damage accrual. Mean ESSDAI reduced from 6.4 (±3.7) at baseline to 4.9 (±5.4) and mean CCI increased from 1.6 (±0.8) to 3.5 (±1.6) at 20 years. At univariate analysis, MHD patients had significant higher median ESSDAI at all timepoints and CCI at 5 and 20 years, higher frequency of parotid swelling at 5 years (35% vs 7%, p=0.021), low C4 (30% vs 3%, p=0.012) at 15 years and monoclonal component at 20 years (30% vs 7%, p=0.047) and lower of arthritis at 10 years (0% vs 23%, p=0.033) in comparison to LND. Patients with MHD had higher prevalence of glucocorticoid (GC) use at 20 years in comparison to LND (75% vs 27%, p=0.001). Multivariable logistic regression analysis model was able to correctly classify 87.5% of patients. None of the included parameters was independent predictor of outcome variable, likely due to a non-linear relationship among variables and the low subject number included. <h3>Conclusion</h3> This is the first study exploring disease damage at 20 year follow-up in SS patients. Notably, damage occurs early, increases already in the first 5-10 years and is mainly driven by oral and ocular damage, PN involvement and lymphoma. Higher disease activity at baseline and during follow-up and higher GC use, also reflecting a more active disease, represent two important variables contributing to damage. Thus, controlling disease activity since diagnosis and minimizing steroid prescription may reduce long-term damage accrual. Among disease-related features, appearance of parotid swelling in the first 5 years requires awareness. Surely, further studies are needed to identify, at diagnosis, patient phenotype at higher risk of long-term damage. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.